Judson Marc A
a Department of Medicine, Division of Pulmonary and Critical Care Medicine , Albany Medical College , Albany , NY , USA.
Expert Rev Respir Med. 2017 Feb;11(2):111-118. doi: 10.1080/17476348.2017.1281745. Epub 2017 Jan 20.
Most of the morbidity and mortality resulting from pulmonary sarcoidosis relates to complications of fibrotic disease. Because the fibrosis related to pulmonary sarcoidosis is often of minimal clinical importance, pharmacotherapy is not mandated. However, a small fraction of pulmonary sarcoidosis patients develop significant lung fibrosis, and they could potentially benefit from anti-sarcoidosis therapy. A reliable algorithm to determine the likelihood of a pulmonary sarcoidosis patient developing fibrosis would minimize the toxicity of therapy and potentially prevent serious complications of the disease. Areas covered: The mechanisms of fibrosis in pulmonary sarcoidosis are discussed. Granulomatous inflammation is the major cause of fibrosis in pulmonary sarcoidosis. Known risk factors for the development of persistent and fibrotic sarcoidosis, including genetic risk factors are explored. Expert opinion/commentary: Currently, methods to determine the propensity of a pulmonary sarcoidosis to develop significant fibrosis are unreliable. This is an important unmet medical need.
肺部结节病导致的大多数发病和死亡都与纤维化疾病的并发症有关。由于与肺部结节病相关的纤维化通常在临床上重要性极小，因此不强制进行药物治疗。然而，一小部分肺部结节病患者会出现显著的肺纤维化，他们可能会从抗结节病治疗中获益。一种可靠的算法来确定肺部结节病患者发生纤维化的可能性，将使治疗的毒性最小化，并有可能预防该疾病的严重并发症。涵盖领域：讨论了肺部结节病中纤维化的机制。肉芽肿性炎症是肺部结节病中纤维化的主要原因。探讨了已知的持续性和纤维化结节病发生的风险因素，包括遗传风险因素。专家意见/评论：目前，确定肺部结节病发展为显著纤维化倾向的方法并不可靠。这是一个重要的未满足的医疗需求。
